本文選題：腎小球微小病變 + 腎病綜合征�。� 參考：《上海交通大學(xué)》2014年碩士論文

【摘要】：腎小球微小病變（minimal change disease，MCD）是腎病綜合征（NS）常見(jiàn)的一種病理類(lèi)型，約70%~90%小于10歲的兒童NS、約50%10歲以上的兒童NS為MCD，是兒童NS最常見(jiàn)病理類(lèi)型。成人MCD的發(fā)病率較兒童低，報(bào)道約10%-37.4%的成人NS為MCD。與兒童MCD相比，有關(guān)成人尤其中國(guó)成人MCD的臨床病理特點(diǎn)的報(bào)道遠(yuǎn)遠(yuǎn)要少，對(duì)于成人MCD，還有很多方面未被認(rèn)識(shí)，也不明確中國(guó)成人MCD的臨床病理表現(xiàn)與其他種族的報(bào)道是否相似。 本文收集了上海交通大學(xué)醫(yī)學(xué)院附屬瑞金醫(yī)院腎臟科2009年1月至2013年6月間臨床符合NS腎活檢病理診斷為MCD、年齡18歲的162例成人患者的病例資料和隨訪數(shù)據(jù)，結(jié)果顯示162例患者，男性98例，女性64例，男女比例1.53:1，平均年齡33.8±14.8歲（18~76歲），平均發(fā)病年齡33.114.7歲，男性明顯早于女性患者，18-30歲年齡段的患者占總數(shù)的54.3%，男性患者主要集中在18-30歲，高達(dá)66.3%，女性患者主要分布在18-30歲及31-40歲，所占比例分別為35.9%和31.3%。162例患者均表現(xiàn)為不同程度的水腫、大量蛋白尿、低白蛋白血癥伴高脂血癥，47例（29.0%）患者發(fā)病時(shí)存在不同誘因，以感染最為常見(jiàn)（61.7%），僅17.9%的患者尿蛋白定量4g/24h，45.7%的患者尿蛋白定量介于4-8g/24h，36.4%的患者尿蛋白定量8g/24h。28.4%的患者伴有鏡下血尿，17.3%的患者血清肌酐115umol/L，以MDRD公式估算腎小球?yàn)V過(guò)率（eGFR），31.5%的患者eGFR在60-90ml/min/1.73m2，13.6%和8.0%的患者eGFR為30-60ml/min/1.73m2和30ml/min/1.73m2，而以EPI公式計(jì)算，15.4%的患者eGFR介于60-90ml/min/1.73m2，4.9%和8.0%的患者EPI-eGFR介于30-60ml/min/1.73m2和30ml/min/1.73m2，60歲以上eGFR均90ml/min/1.73m2，隨著年齡的增高腎功能受累的比例越高。10例（6.2%）患者合并高血壓病，2例合并2型糖尿病，其中1例患者為糖尿病合并高血壓。18-30歲與31-60歲年齡組、初發(fā)和復(fù)發(fā)組、激素敏感和激素依賴(lài)組相比，，兩組在24h尿蛋白定量、血漿白蛋白、腎功能水平方面均未發(fā)現(xiàn)統(tǒng)計(jì)學(xué)差異。 對(duì)162例成人MCD腎組織病理改變進(jìn)行統(tǒng)計(jì)發(fā)現(xiàn)，腎小球球性硬化、腎間質(zhì)纖維化、腎間質(zhì)炎細(xì)胞浸潤(rùn)、腎小管萎縮、腎血管病變的比率分別為32.7%、42.6%、55.6%、45.7%、37.0%，除腎間質(zhì)炎細(xì)胞浸潤(rùn)，余病理改變的比率均隨著年齡的增長(zhǎng)而增加。經(jīng)年齡糾正后，腎小球球性硬化的比率與年齡的關(guān)聯(lián)消失。161例標(biāo)本腎間質(zhì)纖維化和腎小管萎縮程度均為輕度，96.7%伴有腎間質(zhì)炎細(xì)胞浸潤(rùn)的標(biāo)本其炎細(xì)胞浸潤(rùn)程度為輕度，其余為中度，部分標(biāo)本炎細(xì)胞浸潤(rùn)程度重于腎小管萎縮及腎間質(zhì)纖維化。11例（6.8%）患者的腎組織標(biāo)本合并急性腎小管壞死，該患者均有腎功能受累，血肌酐418.5±265.8umol/L（143~956umol/L）。11例患者中2例年齡30歲（2.3%），7例（11.5%）介于31-60歲之間，2例（14.3%）為60歲或60歲以上。90%（9/10例）伴有高血壓的MCD患者可見(jiàn)腎血管病變。初發(fā)和復(fù)發(fā)組、激素敏感和激素依賴(lài)組相比，兩組腎臟病理改變上無(wú)統(tǒng)計(jì)學(xué)差異。88.3%的標(biāo)本免疫熒光均為陰性，僅少部分標(biāo)本可見(jiàn)系膜區(qū)IgM（8.0%）、IgA（7.4%）或C3（3%）弱陽(yáng)性或陽(yáng)性，局灶節(jié)段或彌漫節(jié)段沉積。所有電鏡標(biāo)本均可見(jiàn)足細(xì)胞足突彌漫融合，2例合并薄基底膜病。 綜上所述，本研究通過(guò)入選大組的成人MCD患者，總結(jié)了其臨床及病理特征，發(fā)現(xiàn)男性多于女性，發(fā)病年齡男性早于女性；感染為MCD最常見(jiàn)的誘發(fā)因素，伴發(fā)鏡下血尿少；合并高血壓，糖尿病亦可并發(fā)MCD，腎小球球性硬化、腎間質(zhì)纖維化、腎小管萎縮、腎血管病變的發(fā)生率和嚴(yán)重程度隨年齡增長(zhǎng)而增長(zhǎng)，腎血管病變與年齡和高血壓均有關(guān)，部分患者可合并急性腎功能損傷，甚至引起急性腎小管壞死，尤其老年60歲患者，更易并發(fā)，極少數(shù)患者可合并薄基底膜病。初發(fā)與復(fù)發(fā)、激素敏感與激素依賴(lài)兩組的臨床病理改變無(wú)明顯差異，提示依據(jù)臨床病理改變不能判斷對(duì)單純激素治療的反應(yīng)。
[Abstract]:Minimal change disease (MCD) is a common pathological type of nephrotic syndrome (NS), about 70%~90% less than 10 years old in children, and NS in children above the age of 10, and NS is MCD in children aged over 50%10 years. The clinicopathological features of Chinese adult MCD, especially in Chinese adults, are far from being reported. For adult MCD, many aspects are not known, nor are the clinicopathological manifestations of Chinese adult MCD similar to those of other ethnic groups.
The case data and follow-up data of 162 adult patients, aged 18 years old, were collected from the Department of Nephrology, Ruijin Hospital Affiliated to Shanghai Jiaotong University School of Medicine, from January 2009 to June 2013. The results showed that 162 patients, 98 males and 64 females, male and female ratio 1.53:1, and the average age of 33.8 + 14.8 years (18~). At the age of 76), the average age of onset was 33.114.7 years old, the male was earlier than the female, and the 18-30 year old patients accounted for 54.3% of the total. The male patients were mainly concentrated at 18-30 years old and 66.3%. The female patients were mainly distributed at 18-30 and 31-40 years old. The proportion of patients in the proportion of 35.9% and 31.3%.162, respectively, showed to varying degrees of edema and a large amount of eggs. White urine, hypoalbuminemia with hyperlipidemia, 47 cases (29%) have different causes, the most common (61.7%) infection (61.7%), only 17.9% of the urine protein quantitative 4g/24h, 45.7% of patients with urine protein quantitative 4-8g/24h, 36.4% of the urine protein quantitative 8g/ 24h.28.4% patients with microscopic hematuria, 17.3% of the patients with serum creatinine 115um Ol/L, the glomerular filtration rate (eGFR) was estimated with the MDRD formula. In 31.5% of patients with eGFR in 60-90ml/min/1.73m2,13.6% and 8%, eGFR was 30-60ml/min/1.73m2 and 30ml/min/1.73m2, and in EPI formula, 15.4% of patients eGFR were between 60-90ml/min/1.73m2,4.9% and 8%. The higher eGFR was 90ml/min/1.73m2, the higher the proportion of renal function increased with age.10 (6.2%) patients with hypertension, 2 cases with type 2 diabetes, of which 1 patients with diabetes combined with hypertension and 31-60 years of age, primary and recurrent groups, Ji Sumin and hormone dependence group, the two group in the 24h urine protein quantitative, blood No statistical difference was found in plasma albumin and renal function.
The pathological changes of renal tissue in 162 adult MCD cases showed that glomerular sclerosis, renal interstitial fibrosis, renal interstitial inflammation, renal tubule atrophy, renal tubule atrophy and renal vascular lesions were 32.7%, 42.6%, 55.6%, 45.7%, 37% respectively, except the infiltration of renal interstitial inflammatory cells, and the ratio of the remaining pathological changes increased with age. After correction, the incidence of glomerular sclerosis was associated with age. The degree of renal interstitial fibrosis and renal tubule atrophy were mild in.161 specimens. 96.7% of the specimens accompanied by infiltration of renal interstitial cells were mild, the other was moderate, and the degree of infiltration of some specimens was heavier than that of renal tubule atrophy and renal interstitial fiber. Renal tissue specimens of.11 cases (6.8%) were combined with acute renal tubular necrosis. The patients had renal function involvement, 2 cases of blood creatinine 418.5 + 265.8umol/L (143~956umol/L).11 patients were 30 years old (2.3%), 7 cases (11.5%) were between 31-60 years, 2 (14.3%) was 60 years old or 60 years older than.90% (9/10 cases) with hypertension and MCD patients showed renal vessels. In the initial and recurrent groups, compared with the hormone sensitivity and hormone dependence group, there was no statistical difference in the pathological changes of the two groups. The immunofluorescence of the specimens of.88.3% was negative, only a few specimens were found in the mesangial region IgM (8%), IgA (7.4%) or C3 (3%) were weakly positive or positive, and the focal segmental or diffuse segments were deposited. All the electron microscopic specimens showed podocyte. The foot process was filled with diffuse fusion, and 2 cases with thin basilar membrane disease.
To sum up, this study summed up the clinical and pathological features of adult MCD patients in a large group of adults, and found that males were more than women and were older than women; infection was the most common inducing factor of MCD, with less hematuria, combined hypertension and MCD, glomerular sclerosis, renal interstitial fibrosis, and kidney. The incidence and severity of renal vascular lesions increase with age. Renal vascular lesions are associated with age and hypertension. Some patients can merge acute renal damage and even cause acute renal tubular necrosis. In particular, the elderly 60 year old patients are more likely to be complicated. A few patients can combine thin basilar membrane disease. There was no significant difference in clinicopathological changes between the two groups of hormone sensitivity and hormone dependence, suggesting that the response to simple hormone therapy can not be judged according to clinical pathological changes.
【學(xué)位授予單位】：上海交通大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2014
【分類(lèi)號(hào)】：R692.6

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